: Heart and autologous stem cells transplantation in AL amyloidosis.

MARIA LOURDES POSADAS MARTINEZ

Simposio; XVIIIth International Symposium on Amyloidosis; 2022

Contribution ID : 126Track / Type : ISA 2022 Abstract SubmissionFormat : Undecided (Oral or Poster Presentation)Title : Heart and autologous stem cells transplantation in AL amyloidosis.Author(s) : Aguirre, María Adela; Carretero, Marcelina; Faelo, Franco; Villanueva, Eugenia; Brulc, Erika Bárbara; Sáez, María Soledad; Sorroche, Patricia Beatriz; Belziti, Cesar; Perez de Arenaza, Diego; Marenchino, Ricardo; Posadas Martínez, María Lourdes; Nucifora, Elsa Mercedes Background: Seventy percent of AL amyloidosis patients develop cardiac involvement, which is a key prognostic factor. Heart transplantation and subsequent induction chemotherapy and autologous stem cell transplantation (ASCT) are treatment possibilities for selected patients. Objective: To describe the evolution of serum light chain between heart transplantation and autologous stem cell transplantation. Materials & Methods: Case series of consecutive patients diagnosed as AL systemic amyloidosis who underwent a heart and autologous transplantation from the Institutional Amyloidosis Registry (RIA) of the Italian Hospital of Buenos Aires, between January 2010 and November 2021. The quantitative variables were described with their median and interquartile range and the categorical variables as absolute and relative frequencies. Overall and disease-free survival was estimated using Kaplan-Meier.Results & Discussion: Among 106 patients with AL amyloidosis, 18 had a heart transplantat and 6 had a heart trasplant followed by chemotherapy induction and ASCT. All patients with ASCT were younger than 70 years, with a serum creatinine value < 1.5 mg/ml, ejection fraction > 45%, with normal respiratory function tests and menos de dos órganos comprometidos. Five patients had light chain data available. In the period between transplants, the light chain involved decreased in 3 patients and remained stable in 2. The median time between heart transplantation and ASCT was 344 days (IR 317-582 days). All patients received low-dose CyBorD regimen from disease diagnosis to heart transplantation and then resumed the same full-dose regimen three months after heart transplantation and until the ASCT. Tacrolimus and mycophenolate mofetil were the immunosuppressive regimen used after heart transplantation. The 2-year overall survival rate and progression-free survival rate were 100%. At the end of follow-up, none patient relapsed in the solid organ transplantation.In the present study, we found that no patient showed disease progression in the period between transplants. Our results are similar to the study by Renteria et al., [1] in which only 1 patient out of the 12 analyzed showed disease progression in the period between transplants, with a median time between transplants of 6 months, and without chemotherapy between transplants. However, our patients had a median between transplants of 11 months, with induction chemotherapy established between these periods, which supports our results. We want to highlight that two of the patients had a delay in receiving the ASCT due to difficulties in accessing the care center as a consequence of the preventive and mandatory social isolation imposed in our country by the SaRs-CoV 2 pandemic. This could explain the greater time between transplants when compared to the study by Ranteri et al.Summary & Conclusions: Light chains decreased or remained stable in all cases. Immunosuppressive therapy after heart transplantation may have some effects on clonal plasma cells, but the effect is unclear. This work may lead to future studies on the effects of immunosuppressants such as tacrolimus and mycophenolate mofetil on plasma cells.Key words: Immunoglobulin Light chain Amyloidosis; Treatment; Organ Transplantation; Stem Cell Transplantations